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Fitzpatrick's Dermatology, 9th Edition (Chapter-based Decks) > 134: Cutaneous Paraneoplastic Syndromes > Flashcards

134: Cutaneous Paraneoplastic Syndromes Flashcards

1
Q

What are the characteristics of Acanthosis Nigricans as a cutaneous marker?

A

Acanthosis Nigricans is characterized by symmetric hyperpigmentation, thickening and increased skin markings, commonly occurring in skinfold areas. Most common locations include axillae, neck, external genitalia, groin, face, inner thighs, antecubital and popliteal areas, umbilicus, and perianal regions.

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2
Q

What is the clinical significance of malignant Acanthosis Nigricans?

A

Malignant Acanthosis Nigricans can occur before, during, or after the detection of cancer and may indicate a potential association with neoplastic diseases.

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3
Q

What are the management strategies for Acanthosis Nigricans?

A

Management strategies include identifying underlying malignancy, topical keratolytics, oral retinoids, dietary interventions, cyproheptadine, and cosmetic treatments.

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4
Q

What are the common differential diagnoses (DDX) for Acanthosis Nigricans?

A

Common differential diagnoses include psoriasiform dermatitis, atopic dermatitis, pellagra, infections, and various pigmentary disorders.

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5
Q

What are cutaneous paraneoplastic syndromes?

A

Remote effects of underlying neoplastic diseases that are not direct effects of metastases or tumor invasion.

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6
Q

What is the significance of insulin in benign cutaneous paraneoplastic syndromes?

A

Insulin plays a significant role through IGF-1.

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7
Q

What are the clinical features of Acanthosis Nigricans?

A

Symmetric hyperpigmentation, thickening, and increased skin markings, often presenting as hyperpigmented velvety plaques.

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8
Q

What is the most common location for Acanthosis Nigricans?

A

Axillae, neck, external genitalia, groin, face, inner thighs, antecubital and popliteal areas, umbilicus, and perianal region.

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9
Q

What is the relationship between Acanthosis Nigricans and malignancy?

A

Malignant Acanthosis Nigricans can occur before, during, or after detection of cancer and parallels the course of the underlying malignancy.

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10
Q

What are some management strategies for Acanthosis Nigricans?

A

Identify underlying malignancy, use topical keratolytics, dietary fish oil, metformin, and other treatments like cyproheptadine and laser therapy.

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11
Q

What is the role of TGF alpha in malignant cutaneous paraneoplastic syndromes?

A

The tumor produces TGF alpha, stimulating keratinocyte proliferation and improving or resolving following treatment of underlying malignancy.

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12
Q

What are some differential diagnoses for Acanthosis Nigricans?

A

Psoriasiform dermatitis, atopic dermatitis, pellagra, infections like candidiasis, and other pigmentary disorders.

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13
Q

What is the typical appearance of the skin in Acanthosis Nigricans?

A

Darker skin pigmentation with a dirty appearance, thickening, and increased skin markings leading to hyperpigmented velvety plaques.

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14
Q

A patient presents with sudden, extensive acanthosis nigricans involving mucosal areas. What malignancy should be suspected?

A

The malignancy to suspect is intraabdominal adenocarcinoma, with gastric cancer being the most common.

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15
Q

What is the most common neoplasm associated with Acquired Ichthyosis?

A

Hodgkin disease is the most common neoplasm associated with Acquired Ichthyosis.

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16
Q

What are the clinical features of Tripe Palms?

A

Rough, thickened, and velvety palms with exaggerated dermatoglyphics, often coexisting with acanthosis nigricans.

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17
Q

What underlying conditions are associated with Pityriasis Rotunda?

A

Pityriasis Rotunda is associated with chronic diseases, infections, and malignancies, most commonly gastrointestinal hepatocellular cancer and hematologic malignancies.

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18
Q

What is the significance of sudden onset ichthyosis in adults?

A

Sudden onset of ichthyosis in an adult may be a presenting sign of underlying malignancy.

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19
Q

What is the management approach for Tripe Palms?

A

The management approach includes complete malignancy workup, radiographic evaluation, treating underlying malignancy, and potential improvement with oral retinoids.

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20
Q

What are the histopathological findings in Tripe Palms?

A

Histopathological findings include hyperkeratosis, acanthosis, papillomatosis, and may include dermal mucin and mast cells.

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21
Q

What is the typical demographic for Type 1 pityriasis rotunda?

A

Black or East Asian individuals.

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22
Q

What is the typical demographic for Type 2 pityriasis rotunda?

A

White individuals.

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23
Q

A patient with Hodgkin disease develops diffuse, symmetrical scaling on the trunk and extremities. What is the likely cutaneous paraneoplastic syndrome?

A

The likely syndrome is acquired ichthyosis, which is associated with Hodgkin disease and other malignancies.

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24
Q

A patient presents with tripe palms and acanthosis nigricans. What malignancy is most likely associated with this presentation?

A

The most likely associated malignancy is gastric cancer.

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25
Q

A patient with tripe palms has pulmonary cancer. What is the specific subtype of lung cancer most commonly associated with this condition?

A

The specific subtype is squamous cell carcinoma of the lung.

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26
Q

What is the Leser-Trélat sign and its clinical significance?

A

The Leser-Trélat sign is characterized by a rapid increase in the number and size of seborrheic keratoses, often associated with internal malignancy.

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27
Q

What are the common clinical features associated with the Leser-Trélat sign?

A

Common features include numerous eruptive seborrheic keratoses, pruritus in about 50% of cases, and co-occurrence with other hyperkeratotic paraneoplastic dermatoses.

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28
Q

What are the management options for the Leser-Trélat sign?

A

Management may include α-hydroxy acids, retinoids, TCA, cryosurgery, dermabrasion, laser therapy, and shave removal.

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29
Q

What is the prognosis for patients with the Leser-Trélat sign?

A

The prognosis is generally poor, with more than 50% having metastatic tumors at the time of diagnosis.

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30
Q

What is the relationship between the eruption of seborrheic keratoses and underlying malignancy in Leser-Trélat sign?

A

The eruption parallels the course of the underlying malignancy in some cases.

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31
Q

What are the histopathologic findings of seborrheic keratoses in the context of the Leser-Trélat sign?

A

Findings include epidermal hyperkeratosis, papillomatosis, and acanthosis.

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32
Q

What benign conditions can mimic the Leser-Trélat sign?

A

Pseudosign of Leser-Trélat can occur in conditions like pregnancy, HIV, heart transplantation, and acromegaly.

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33
Q

What is the significance of cooccurrence with other hyperkeratotic paraneoplastic dermatoses in Leser-Trélat sign?

A

Cooccurrence is common, with about one-third of patients having acanthosis nigricans, indicating potential malignancy.

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34
Q

A patient has a sudden increase in the number and size of seborrheic keratoses. What malignancy should be suspected?

A

This phenomenon is called the Leser-Trelat sign, most commonly associated with gastrointestinal tract malignancies.

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35
Q

What are the characteristic clinical features of Acrokeratosis Paraneoplastica of Bazex?

A

Symmetrical erythematous to violaceous scaly patches or plaques over acral extremities, ears, and bridge of the nose.

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36
Q

What is the immunologic mechanism involved in Acrokeratosis Paraneoplastica of Bazex?

A

Antibodies against the tumor crossreact with keratinocyte and basement membrane antigens, with a T cell-mediated immune response.

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37
Q

What are the stages of Acrokeratosis Paraneoplastica of Bazex and their characteristics?

A

Stages include: 1st - ill-defined scaly papule; 2nd - extensive red-to-purple scaly plaques; 3rd - papulosquamous lesions on other sites.

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38
Q

What is the management approach for Acrokeratosis Paraneoplastica of Bazex?

A

Treatment of the primary neoplasm is the most effective therapy.

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39
Q

What is the most common associated malignancy with Acrokeratosis Paraneoplastica of Bazex?

A

Squamous cell carcinomas of the upper aerodigestive tract.

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40
Q

What is the first sign of nail involvement in Acrokeratosis Paraneoplastica?

A

Paronychia.

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41
Q

What is the typical skin eruption in the first stage of Acrokeratosis Paraneoplastica?

A

Ill-defined scaly papule, nonspecific dermatitis, symmetrical, asymptomatic.

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42
Q

What are the common sites of involvement in Acrokeratosis Paraneoplastica?

A

Nails (77%), ears (76%), fingers (65%), nose (62%), palms (56%), hands (51%), and soles (49%).

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43
Q

What is the management approach for Acrokeratosis Paraneoplastica?

A

Treatment of the primary neoplasm is the most effective therapy.

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44
Q

What immunologic mechanism is involved in Acrokeratosis Paraneoplastica?

A

Antibodies against the tumor crossreact with keratinocyte and basement membrane antigen.

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45
Q

What is the prognosis for skin eruptions in Acrokeratosis Paraneoplastica?

A

Skin eruption can occur before, simultaneously, or after the diagnosis of the neoplasm, and lesions typically resolve after successful treatment of the underlying malignancy.

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46
Q

What are the characteristics of the skin eruption in the second stage of Acrokeratosis Paraneoplastica?

A

More extensive, red-to-purple scaly plaques, with palms and soles developing keratoderma.

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47
Q

What is a distinguishing clinical feature of Acrokeratosis Paraneoplastica?

A

Involvement of the helices of the ears and the tip of the nose.

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48
Q

What is the likely diagnosis for a male patient over 40 years old with symmetrical erythematous scaly patches on the ears and nose, and what malignancy should be investigated?

A

The likely diagnosis is acrokeratosis paraneoplastica of Bazex, and squamous cell carcinoma of the upper aerodigestive tract should be investigated.

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49
Q

What is the significance of adult-onset dermatomyositis (DM) in relation to malignancy?

A

Adult-onset dermatomyositis (DM) is significantly associated with malignancy, with a risk of 18-25%. The risk is highest in the first year after diagnosis, then steadily declines but remains slightly elevated even after 5 years.

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50
Q

What are the predictive signs of accompanying malignancy in adult-onset dermatomyositis?

A

Predictive signs include cutaneous necrosis, cutaneous vasculitis, lack of interstitial lung disease, dysphagia, male gender, elevated erythrocyte sedimentation rate, and older age at onset.

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51
Q

What is the relationship between progressive systemic sclerosis (PSS) and cancer risk?

A

Progressive systemic sclerosis (PSS) is associated with an increased risk of cancer, particularly within the first 12 months after diagnosis.

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52
Q

What type of cancer is most frequently associated with progressive systemic sclerosis?

A

Lung cancer is the most frequent type of cancer associated with progressive systemic sclerosis.

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53
Q

What are the characteristics of Erythema Gyratum Repens?

A

Numerous serpiginous bands arranged in a parallel configuration of concentric red swirls, a ‘wood-grain’ appearance, fine scale along the trailing edge of erythema, rapid migration rate of lesions, common sparing of hands, feet, and face, with occasional volar hyperkeratosis, and pruritus present in many cases.

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54
Q

What is the clinical significance of the eruption associated with Erythema Gyratum Repens?

A

The eruption is clinically significant as it is associated with underlying malignancy in 70% of cases.

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55
Q

What is the estimated rate at which lesions of Erythema Gyratum Repens migrate?

A

The lesions migrate at an estimated rate of 1 cm per day.

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56
Q

What treatments are recommended for Erythema Gyratum Repens?

A

Treatment includes locating and treating the primary malignancy, topical steroids, vitamin A, and azathioprine.

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57
Q

What is the significance of anti-p155 antibodies in adult patients with dermatomyositis?

A

Anti-p155 antibodies are a promising cancer marker in adult patients with dermatomyositis.

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58
Q

What malignancies should be screened for in patients with elevated anti-p155 antibodies?

A

Malignancies to screen for include lung, breast, ovarian, cervical, pancreatic, gastric, colorectal, and prostate cancers.

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59
Q

What is the most commonly associated hematologic malignancy with Sweet syndrome?

A

Acute myeloblastic leukemia.

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60
Q

What are the atypical features of Pyoderma Gangrenosum (PG) in patients with underlying hematologic disease or malignancy?

A

Atypical features include abrupt onset, superficial lesions, hemorrhagic bullae, and involvement of the upper extremities.

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61
Q

What are the mainstay treatments for malignancy-associated Sweet syndrome?

A

Systemic corticosteroids and other standard treatments used in classical or idiopathic Sweet syndrome.

62
Q

What is Multicentric Reticulohistiocytosis and its association with malignancies?

A

A rare, systemic disease presenting with destructive polyarthritis and typical cutaneous lesions, with 25% of patients having associated malignancies.

63
Q

What is the prognosis for patients with primary systemic amyloidosis (AL amyloidosis) if left untreated?

A

The prognosis is generally poor, with a median survival time of 12 months.

64
Q

What is the characteristic clinical finding in paraneoplastic pemphigus?

A

Painful, hemorrhagic oral erosions.

65
Q

What is the frequency of associated malignancy in patients with dermatitis herpetiformis?

A

Up to 4.3%, predominantly in males.

66
Q

What is the typical presentation of Bullous Pemphigoid?

A

Intact skin blisters and pruritus in elderly patients.

67
Q

What is Necrobiotic Xanthogranuloma associated with?

A

Hematologic disorders and monoclonal gammopathy.

68
Q

What are the common skin manifestations of Scleromyxedema?

A

Papular mucinosis with a predilection for the face, arms, and hands.

69
Q

What is the most common associated plasma cell dyscrasia with necrobiotic xanthogranuloma?

A

Monoclonal gammopathy of undetermined significance, along with smoldering multiple myeloma and multiple myeloma.

70
Q

What is the characteristic appearance of papules and nodules in Multicentric Reticulohistiocytosis?

A

A characteristic ‘coral bead’ appearance of periungual papules.

71
Q

What is the treatment approach for underlying malignancy in patients with Pyoderma Gangrenosum?

A

Improvement of bullous PG with treatment of the underlying malignancy.

72
Q

What is the most commonly associated malignancy with AL amyloidosis?

A

Multiple myeloma.

73
Q

What is the prognosis for patients with malignancy-associated Paraneoplastic Pemphigus?

A

Prognosis is poor.

74
Q

What skin condition is associated with gluten sensitivity?

A

Dermatitis Herpetiformis.

75
Q

What should be performed to rule out thyroid dysfunction in Scleromyxedema?

A

Serum protein electrophoresis in conjunction with thyroid studies.

76
Q

What is the likely diagnosis for a patient with painful, hemorrhagic oral erosions and polymorphous skin eruptions?

A

The likely diagnosis is paraneoplastic pemphigus, and lymphoproliferative diseases such as Hodgkin lymphoma and chronic lymphocytic leukemia should be considered.

77
Q

What malignancy is most commonly associated with systemic amyloidosis presenting with macroglossia and waxy skin thickening?

A

Multiple myeloma.

78
Q

What hematologic disorder is most commonly associated with necrobiotic xanthogranuloma?

A

Monoclonal gammopathy of undetermined significance (MGUS).

79
Q

What malignancy should be considered in a patient with bullous pemphigoid, particularly in Japanese populations?

A

In Japanese populations, there is a higher risk of malignancy, and cancer screening should be considered.

80
Q

What lymphoproliferative diseases are most commonly associated with paraneoplastic pemphigus?

A

Hodgkin lymphoma, chronic lymphocytic leukemia, and Castleman disease.

81
Q

What diagnostic tests should be performed to rule out multiple myeloma?

A

Tests include serum and urine protein electrophoresis, immunofixation electrophoresis, serum free light chain assay, bone marrow biopsy, and skeletal imaging.

82
Q

What lymphoproliferative diseases are most commonly associated with paraneoplastic pemphigus?

A

The most commonly associated diseases are Hodgkin lymphoma, chronic lymphocytic leukemia, and Castleman disease.

83
Q

What is the most common clinical manifestation of Trousseau Syndrome?

A

The most frequent clinical manifestation of Trousseau Syndrome is venous thromboembolism (VTE), which is the second most common cause of death in patients with cancer.

84
Q

What are the common malignancies associated with Hypertrichosis Lanuginosa Acquista?

A

The most commonly associated malignancies are lung cancer, colorectal cancer in men, colorectal cancer followed by lung and breast cancer in women, and other cancers including ovary, uterus, urinary bladder carcinoma, lymphoma, and leukemia.

85
Q

What is Virchow’s triad and its relevance in Trousseau Syndrome?

A

Virchow’s triad consists of three factors that contribute to thrombosis: abnormal blood flow or stasis, vessel wall injury, and blood hypercoagulability. In Trousseau Syndrome, these factors are often present due to cancer-associated hypercoagulability, leading to increased risk of venous thromboembolism.

86
Q

What is the first line treatment for Trousseau Syndrome?

A

The first line treatment for Trousseau Syndrome is low-molecular-weight heparin, which is recommended alongside treatment of the underlying malignancy.

87
Q

How does Hypertrichosis Lanuginosa Acquista develop and what are its clinical features?

A

Hypertrichosis Lanuginosa Acquista develops with a relatively sudden appearance of long, fine, nonpigmented lanugo hairs, primarily on the face and ears. It may be accompanied by glossitis, hypertrophy of tongue papillae, oral hyperpigmentation, disturbances of taste and smell, acanthosis nigricans, diarrhea, adenopathy, and weight loss.

88
Q

What is Hypertrichosis Lanuginosa Acquista associated with?

A

It is associated with pancreatic carcinoma and presents as a sudden appearance of long, fine, nonpigmented lanugo hairs.

89
Q

What is the risk of thrombosis in patients with cancer compared to the normal population?

A

Patients with cancer have a 4 to 7 times higher risk of thrombosis than the normal population.

90
Q

What are some unusual sites for thrombosis in patients with malignancy?

A

Upper limbs, thrombosis of visceral organs, and brain.

91
Q

What is the significance of neutrophil extracellular traps (NETs) in Trousseau Syndrome?

A

NETs have effects on the intrinsic coagulation pathway and are released as a result of neutrophil programmed cell death (NETosis).

92
Q

What should be included in the diagnostic evaluations for occult malignancy in patients with Hypertrichosis Lanuginosa Acquista?

A

Evaluations should focus on the lung, colon, rectum, and breast.

93
Q

What malignancies should be investigated in a patient with sudden, long, fine, nonpigmented lanugo hairs on the face and ears?

A

Malignancies to investigate include lung and colorectal cancer in men, and colorectal, lung, and breast cancer in women.

94
Q

What is the underlying mechanism for venous thromboembolism (VTE) in a patient with pancreatic cancer?

A

The mechanism involves overexpression of tissue factor by tumor cells and neutrophil extracellular traps (NETs). The first-line treatment is low-molecular-weight heparin.

95
Q

What cancers are associated with the highest risk of thrombosis in patients with Trousseau syndrome?

A

Cancers with the highest risk of thrombosis include pancreatic, brain, lung, stomach, and ovarian cancers.

96
Q

What is the recommended treatment for a patient with Trousseau syndrome who has deep vein thrombosis?

A

The recommended treatment is anticoagulant therapy, with low-molecular-weight heparin as the first-line treatment.

97
Q

What malignancies are most commonly associated with cutaneous metastases manifesting as firm, mobile nodules on the thorax?

A

The most commonly associated malignancies are breast cancer in women and lung cancer in men.

98
Q

What malignancy is most commonly associated with alopecia neoplastica?

A

The most commonly associated malignancy is metastatic breast cancer.

99
Q

What malignancy is most commonly associated with pigmented lesions with a bluish tint?

A

The most commonly associated malignancy is metastatic melanoma.

100
Q

What are the common clinical features of leukemia cutis?

A

Leukemia cutis presents as pink, red, and/or brown-to-purple macules, papules, plaques, and nodules that are usually firm and painless.

101
Q

What is the significance of biopsy in the diagnosis of leukemia cutis?

A

Biopsy is essential to confirm the diagnosis of leukemia cutis. It helps in determining the subtype of leukemia through immunohistochemical and molecular genetic methods, which is crucial for targeted treatment and assessing prognosis.

102
Q

How does extramammary Paget disease typically present?

A

Extramammary Paget disease typically presents as a chronic erythematous scaling eruption of the nipple and areola, indicating ductal carcinoma of the underlying breast. It most commonly occurs in the anogenital skin, followed by the axillae and penis.

103
Q

What are the three types of extramammary Paget disease?

A

The three types of extramammary Paget disease are: primary intraepidermal adenocarcinoma (most common type), associated with visceral malignancy (most commonly of the GI or genitourinary systems), and associated with an adjacent sweat gland carcinoma.

104
Q

What is the clinical implication of ‘aleukemic’ leukemia cutis?

A

‘Aleukemic’ leukemia cutis refers to cases where leukemia cutis appears without systemic involvement, often as a presenting manifestation months or years before systemic symptoms develop. This indicates a potential for aggressive disease and a poor prognosis.

105
Q

What are cutaneous manifestations of leukemia and lymphoma referred to as?

A

Leukemia cuts and lymphoma cuts.

106
Q

What is a common clinical feature of leukemia cutis?

A

Pink, red, and/or brown-to-purple macules, papules, plaques, and nodules that are usually firm and painless.

107
Q

What can rapid and generalized eruption of cutaneous lesions suggest in a patient?

A

An acute form of leukemia.

108
Q

What is the most common type of Extramammary Paget disease?

A

Primary intraepidermal adenocarcinoma.

109
Q

Where does Extramammary Paget disease most commonly occur?

A

In the anogenital skin, followed by the axillae and penis.

110
Q

What type of lymphoma is most commonly associated with cutaneous involvement?

A

Systemic anaplastic large-cell lymphoma.

111
Q

What is the treatment approach for cutaneous manifestations of leukemia?

A

Treatment is targeted to the specific type of underlying leukemia.

112
Q

What is a common skin manifestation in adult T-cell leukemia-lymphoma?

A

Skin lesions resembling mycosis fungoides skin lesions.

113
Q

What is the likely diagnosis for a patient with chronic erythematous scaling of the nipple and areola?

A

The likely diagnosis is Paget disease of the breast, and ductal carcinoma of the underlying breast should be investigated.

114
Q

What is the significance of mucosal involvement and gum hypertrophy in leukemia cutis?

A

This presentation is common in leukemia cutis and may indicate aggressive disease and poor prognosis.

115
Q

What is the most common type of malignancy associated with extramammary Paget disease?

A

The most common type of malignancy is primary intraepidermal adenocarcinoma.

116
Q

What does rapid and generalized eruption of cutaneous lesions in leukemia cutis suggest?

A

This suggests an acute form of leukemia, particularly acute myeloid leukemia.

117
Q

What are the unique features associated with Acanthosis nigricans in the context of paraneoplastic dermatoses?

A
  • Older age, rapid course, extensive, and oral involvement
  • Indicate malignancy associations; more common in benign conditions with insulin resistance and metabolic syndromes.
118
Q

What is the percentage of patients with Pityriasis rotunda who have an internal malignancy?

A

30% of Type I patients have an internal malignancy.

119
Q

What is the relationship between Bazex syndrome and internal malignancy?

A

Nearly 100% of patients with Bazex syndrome have an associated internal malignancy, specifically squamous cell carcinoma (SCC) of the upper aerodigestive tract.

120
Q

What are the common internal malignancies associated with Sweet syndrome?

A

Acute myeloid leukemia (AML) is the most common internal malignancy associated with Sweet syndrome, occurring in 20% of cases.

121
Q

What is the significance of Necrolytic erythema in relation to internal malignancy?

A

Necrolytic erythema is nearly 100% associated with glucagonoma (pancreatic alpha cell tumor), and early recognition is crucial as many patients have metastatic tumors at the time of diagnosis.

122
Q

What is the most common internal malignancy associated with Acanthosis nigricans?

A

Adenocarcinoma: intrabdominal/gastric cancer (60%).

123
Q

What is a unique feature of Bazex syndrome?

A

Nearly 100% associated with squamous cell carcinoma of the upper aerodigestive tract.

124
Q

What is the most common internal malignancy associated with Sweet syndrome?

A

Acute myeloid leukemia (AML).

125
Q

What is a unique feature of Dermatomyositis?

A

Older age, males predominate; refractory or flaring of previously well-controlled disease may signal malignancy.

126
Q

What is the most common malignancy associated with Pyoderma gangrenosum?

A

Acute myeloid leukemia (AML).

127
Q

What is the most common condition associated with Scleromyxedema?

A

Monoclonal gammopathy of undetermined significance (MGUS) most common.

128
Q

What is the clinical significance of Systemic amyloidosis?

A

Associated with multiple myeloma; 20% of patients have malignancy.

129
Q

What are the major associated malignancies for Cowden disease?

A

Breast cancer, thyroid cancer, endometrial cancer, hamartomatous intestinal polyps.

130
Q

What is the unique feature of Dermatitis herpetiformis in relation to malignancy?

A

Occurs significantly more often in patients who do not follow a gluten-free diet.

131
Q

Which paraneoplastic dermatosis is associated with nearly 100% of patients having malignancies?

A

Paraneoplastic pemphigus and Hypertrichosis lanuginosa acquista.

132
Q

What is the primary internal malignancy associated with Trousseau syndrome?

A

Pancreatic, brain, and lung cancers.

133
Q

What are the cutaneous findings associated with Gorlin syndrome?

A

Multiple BCCs, epidermoid cysts, milia, and palmar/plantar pits.

134
Q

What genetic syndrome is associated with fibrous dysplasia and trichilemmomas?

A

Cowden syndrome.

135
Q

Which syndrome is linked to multiple BCCs and epidermoid cysts?

A

Gorlin syndrome.

136
Q

What is a major associated internal malignancy of the MEN I syndrome?

A

Angiofibromas, collagenomas, lipomas.

137
Q

What is the major associated internal malignancy with Peutz-Jeghers syndrome?

A

Gastrointestinal cancers, particularly colorectal cancer.

138
Q

What are the characteristic features of Xeroderma pigmentosum (XP)?

A

Photosensitivity, actinic keratosis, multiple skin cancers (BCC, SCC, melanoma).

139
Q

What is the genetic defect associated with Dyskeratosis congenita?

A

DKC1 (X-linked) and TERT (autosomal dominant).

140
Q

What are the minor associated internal malignancies with Beckwith-Wiedemann syndrome?

A

Adrenal cortical tumors, Wilms tumor, hepatoblastoma.

141
Q

What are cutaneous paraneoplastic syndromes?

A

Skin manifestations that are associated with underlying malignancies.

142
Q

What is the genetic defect associated with Neurofibromatosis type 1 (NF1)?

A

NF1 gene mutation.

143
Q

Which syndrome is associated with renal leiomyoma?

A

Hereditary leiomyomatosis and renal cell carcinoma (HLRCC).

144
Q

What are the cutaneous findings associated with Xeroderma pigmentosum (XP)?

A

Photosensitivity, actinic keratosis, lentigines, and poikiloderma.

145
Q

What are paraneoplastic syndromes?

A

Skin manifestations that are associated with underlying malignancies.

146
Q

What is a major associated internal malignancy with Dyskeratosis congenita?

A

Thyroid carcinomas.

147
Q

What is the main characteristic feature of Wiskott-Aldrich syndrome?

A

Eczema, thrombocytopenia, and immunodeficiency.

148
Q

Which syndrome is linked to osteogenic sarcomas?

A

Rothmund-Thomson syndrome.

149
Q

What is a common feature of Beckwith-Wiedemann syndrome?

A

Capillary malformation and macroglossia.

150
Q

What is the internal malignancy associated with Familial atypical multiple mole melanoma syndrome?

A

Melanoma and pancreatic cancer.

151
Q

What are the minor associated internal malignancies with Dyskeratosis congenita?

A

Hodgkin lymphoma and GI carcinoma.

Fitzpatrick's Dermatology, 9th Edition (Chapter-based Decks) (48 decks)

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